Intra-cranial manifestations of the neurocutaneous syndromes

The neurocutaneous syndromes or phakomatoses are a heterogeneous group of congenital disorders primarily involving structures derived from the embryological neuroectoderm. All of the syndromes involve the central nervous system (CNS). Peripheral nerves, skin and other organ systems may also be involved. Twenty to 30 disorders are now classified as neurocutaneous syndromes. This article reviews the intra-cranial imaging features of some of the commonest.